title: Primary antiphospholipid syndrome: a low-grade auto-inflammatory disease?
abstract: OBJECTIVE
  To test the inflammation and immune activation hypothesis in primary thrombotic APS (PAPS) and to identify clinical and laboratory factors related to inflammation and immune activation.
  
  
  METHODS
  PAPS (n = 41) patients were compared with patients with inherited thrombophilia (IT, n = 44) and controls (CTR, n = 39). IgG aCL, IgG anti-beta2-glycoprotein I (beta(2)GPI), high-sensitivity CRP (hs-CRP), serum amyloid A (SAA), CRP bound to oxidized low-density lipoprotein-beta(2)GPI complex (CRP-oxLDL-beta(2)GPI) (as inflammatory markers) neopterin (NPT), soluble CD14 (sCD14) (as immune activation markers) were measured by ELISA.
  
  
  RESULTS
  After correction for confounders, PAPS showed higher plasma levels of hs-CRP (P = 0.0004), SAA (P < 0.01), CRP-oxLDL-beta(2)GPI (P = 0.0004), NPT (P < 0.0001) and sCD14 (P = 0.007) than IT and CTR. Two regression models were applied to the PAPS group: in the first, IgG aCL and IgG beta(2)GPI were included amongst the independent variables and in the second they were excluded. In the first model, SAA (as the dependent variable) independently related to thrombosis number (P = 0.003); NPT (as the dependent variable) independently related to thrombosis type (arterial, P = 0.03) and number (P = 0.04); sCD14 (as the dependent variable) independently related to IgG beta(2)GPI (P = 0.0001), age (0.001) and arterial thrombosis (P = 0.01); CRP-oxLDL-beta(2)GPI (as the dependent variable) independently related to IgG beta(2)GPI (P = 0.0001). In the second model, sCD14 and NPT independently related to each other (P = 0.002) (this was noted also in the IT group, P < 0.0001) and CRP-oxLDL-beta(2)GPI independently predicted SAA (P = 0.002).
  
  
  CONCLUSIONS
  Low-grade inflammation and immune activation occur in thrombotic PAPS and relate to clinical features and aPL levels.
authors.affiliation: Immunoclot Ltd, Leeds LS6 4QT, UK. paxmes@aol.com
authors.forename: P R J
authors.initials: PR
authors.lastname: Ames
authors.forename: I
authors.initials: I
authors.lastname: Antinolfi
authors.forename: A
authors.initials: A
authors.lastname: Ciampa
authors.forename: J
authors.initials: J
authors.lastname: Batuca
authors.forename: G
authors.initials: G
authors.lastname: Scenna
authors.forename: L R
authors.initials: LR
authors.lastname: Lopez
authors.forename: J
authors.initials: J
authors.lastname: Delgado Alves
authors.forename: L
authors.initials: L
authors.lastname: Iannaccone
authors.forename: E
authors.initials: E
authors.lastname: Matsuura
mesh_terms: D000328:Adult; D000368:Aged; D016736:Antiphospholipid Syndrome / Q000276:immunology*; D001323:Autoantibodies / Q000097:blood; D015415:Biomarkers / Q000097:blood; D002097:C-Reactive Protein / Q000032:analysis; D003430:Cross-Sectional Studies; D004797:Enzyme-Linked Immunosorbent Assay / Q000379:methods; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D007249:Inflammation / Q000276:immunology*; D018950:Lipopolysaccharide Receptors / Q000097:blood; D008297:Male; D008875:Middle Aged; D019798:Neopterin / Q000097:blood; D000685:Serum Amyloid A Protein / Q000032:analysis; D019851:Thrombophilia / Q000276:immunology; D053482:beta 2-Glycoprotein I / Q000097:blood
chemical_list: D001323:Autoantibodies; D015415:Biomarkers; D007074:Immunoglobulin G; D018950:Lipopolysaccharide Receptors; D000685:Serum Amyloid A Protein; D053482:beta 2-Glycoprotein I; D019798:Neopterin; D002097:C-Reactive Protein